Pulmonary hypertension in sickle cell disease

被引:103
作者
Ataga, KI
Sood, N
De Gent, G
Kelly, E
Henderson, AG
Jones, S
Strayhorn, D
Lail, A
Lieff, S
Orringer, EP
机构
[1] Univ N Carolina, Div Hematol Oncol, Dept Med, Chapel Hill, NC 27599 USA
[2] Ohio State Univ, Dept Med, Columbus, OH 43210 USA
[3] Sidney F Lebauer Med Ctr, Dept Med, Greensboro, NC USA
[4] Moses Cone Hosp, Greensboro, NC USA
[5] Rho Inc, Chapel Hill, NC USA
关键词
D O I
10.1016/j.amjmed.2004.03.034
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
BACKGROUND: Recurrent vaso-occlusive episodes lead to progressive end-organ damage in patients with sickle cell disease. We sought to determine the prevalence of pulmonary hypertension in adult patients with sickle cell disease and to identify factors associated with this life -threatening complication. METHODS: Sixty patients ( greater than or equal to18 years of age; mean [+/-SD] age, 37 +/- 13 years) followed at a University Medical Center were evaluated. They were selected by a systematic sampling of patients presenting to the clinic for routine follow-up visits. All enrolled subjects underwent a clinical examination, Doppler echocardiography, pulmonary function tests, and hematologic tests during a single visit. Pulmonary hypertension was defined using an age- and body mass index-adjusted nomogram. RESULTS: The prevalence of pulmonary hypertension was 30% (18/60). Ten patients had mild pulmonary hypertension (up to 44 mm Hg), 5 had moderate pulmonary hypertension (45 to 74 turn Hg), and 2 had severe pulmonary hypertension (greater than or equal to75 rum Hg). In a logistic regression model, both lower fetal hemoglobin level and lower systolic blood pressure were associated with the presence of pulmonary hypertension. CONCLUSION: We found that the prevalence of pulmonary hypertension in adult patients with sickle cell disease was substantial, particularly in those with lower levels of fetal hemoglobin and lower systolic blood pressure. (C) 2004 by Elsevier Inc.
引用
收藏
页码:665 / 669
页数:5
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